母细胞性浆细胞样树突细胞肿瘤

母细胞性浆细胞样树突细胞肿瘤是一种少见的侵袭性淋巴瘤,2008年正式命名.临床上以皮肤受累为首发症状,表现为挫伤样结节或斑块.该肿瘤易侵犯骨髓及淋巴结,病程凶险,预后差.其肿瘤细胞来源于浆细胞样树突细胞前体细胞,具有独特的免疫表型:CD4、CD56阳性,TdT和CD68部分阳性,还表达浆细胞样树突细胞相关表面标志CD123、TCL1和BDCA-2.但目前其发病机制仍未明确,且无标准治疗方案.     

母细胞性浆细胞样树突细胞肿瘤研究进展

2008年WHO在造血和淋巴系统肿瘤分类中将发生于皮肤,CD56和CD4阳性,并表达CD123,血树突细胞抗原2和4等浆细胞样树突细胞标记的所谓"母细胞性NK细胞淋巴瘤"命名为母细胞性浆细胞样树突细胞肿瘤,认为其起源于浆细胞样树突状细胞,是一种罕见的临床侵袭性淋巴瘤,可发生于各年龄组,多数为中老年男性。临床多有皮肤损害,表现为孤立性、多发性肿块、结节、斑块、红斑,可迅速累及淋巴结、软组织和中枢神经系统,临床过程凶险。组织学有一定特点,确诊依赖对肿瘤细胞的免疫标记。患者最初对化疗可有较好反应,但复发快,复发率高,中位生存时间仅12～14个月。     

母细胞性浆细胞样树突细胞肿瘤

报告1例母细胞性浆细胞样树突细胞肿瘤。患者女,26岁。面部结节、斑块半年。皮肤科检查:右颧部可见约4 cm×6 cm暗红色浸润性斑块,类圆形分叶状,境界清楚,皮损中央破溃。皮损组织病理检查:真皮全层及皮下脂肪间见较多致密的异形淋巴细胞浸润,细胞中等大小,核膜薄,染色质细,核分裂象易见。免疫组化:CD4(+)、CD56(+)、CD123(+)、CD43(+)、CD34(+),Ki-67(60%阳性);CD20、CD79a、丝氨酸蛋白酶(granzyme)B、CD3、CD45RO、CD68、末端脱氧核苷酸转移酶(TDT)、髓过氧化物酶(MPO)、T细胞内抗原(TIA)-1均阴性。诊断:母细胞性浆细胞样树突细胞肿瘤。     

母细胞性浆细胞样树突细胞肿瘤一例并文献复习

本文报道一女性患者,72岁。全身泛发性红色丘疹半年,进行性加重1个月。皮损病理检查示:真皮浅层有一无细胞带,其下单一性弥漫性淋巴样细胞增生浸润,免疫组化检查示:CD56+、CD123+、CD4(弱+)。诊断为母细胞性浆细胞样树突细胞肿瘤。我们对相关文献进行了复习。     

母细胞性浆细胞样树突细胞肿瘤一例北大核心CSCD

患者女,19岁,左下肢暗红色斑丘疹1年余,右腹股沟可触及一樱桃大小淋巴结。皮肤肿物组织病理检查：皮肤正常结构消失,可见淋巴样肿瘤细胞弥漫浸润,细胞中等大小,核仁不清楚;免疫组化：CD4（＋＋）、CD56（＋＋）、CD123（＋＋＋）,TdT部分阳性。骨髓穿刺行流式细胞检查：异常细胞占59．9％,表达CD123st（IL-3Ra）、HLA—DRst、CD56、CD304（BDCA-4）、CD7、CD11b、CD33、CD36、CXCR4和CD13dim,部分细胞表达CD4和CD117。透射电镜观察骨髓细胞：可见大量形态大小均一的淋巴样细胞,细胞表面可见粗大突起,胞核胞质比例高,胞核圆形居中,有呈脑回样深切迹,异染色质块状边集,核仁多见,少量胞质,其中可见内质网、线粒体等细胞器,内质网均呈浆细胞样同心圆状排列。骨髓细胞表现兼具树突细胞及浆细胞特征。诊断：母细胞性浆细胞样树突细胞肿瘤。     

母细胞性浆细胞样树突细胞肿瘤三例临床及病理分析

回顾性分析2014年1月至2019年1月武汉协和医院收治的母细胞性浆细胞样树突细胞肿瘤（BPDCN）3例临床病例特点，并复习相关文献。3例患者 (男2例，女1例) 均以皮肤表现为首发症状，表现为淡红色丘疹、结节、斑块，组织病理均显示真皮及皮下可见弥漫浸润中等大小淋巴样细胞，可见核分裂象，表皮未受累；免疫组化显示CD4，CD56，CD123均阳性。     

母细胞性NK细胞淋巴瘤

母细胞性NK细胞淋巴瘤是一种罕见的侵袭性淋巴瘤,可能来源于浆细胞样树突细胞,与EB病毒感染无关.母细胞性NK细胞淋巴瘤可发生于各年龄组,多数为中老年.临床表现为孤立性、多发性肿块、结节或斑块,可呈挫伤样或形成溃疡.可迅速累及淋巴结、软组织和中枢神经系统,临床过程凶险.组织学有一定特点,免疫组化CD56和CD4阳性.尽管患者最初对各种化疗方案多有较好反应,但均很快复发.发病年龄≤40岁、仅有皮肤损害、>50%的肿瘤细胞表达TdT时,预后相对较好.     

树突细胞亚群与银屑病的相关性研究进展

银屑病是一种病因不明的慢性炎症性、免疫性疾病,其皮损中可发现明显增多的树突细胞和效应性T细胞浸润,提示树突细胞参与了银屑病的炎症反应.正常皮肤组织中,主要分布3类树突细胞:表皮朗格汉斯细胞、真皮髓系树突细胞和浆细胞样树突细胞.在银屑病皮损组织中,3类树突细胞与T细胞、角质形成细胞、中性粒细胞的相互作用可引发和加重皮肤中的炎症反应.此外,浆细胞样树突细胞在以Toll样受体依赖方式分泌的Ⅰ型干扰素还可能促进髓系树突细胞的活化及炎症介质的释放.     

母细胞性浆细胞样树突细胞肿瘤研究及诊疗进展

母细胞性浆细胞样树突细胞肿瘤是一种罕见的侵袭性造血组织肿瘤,病程进展快,预后差。病变常累及皮肤、淋巴结、脾脏、血液和骨髓,其组织病理学与免疫组化具有特征性表现,皮肤损害的组织病理学与皮肤白血病类似,肿瘤细胞呈母细胞性,呈现特征性免疫表型CD4~+CD56~+CD123~+,不表达髓系、T细胞及B细胞特异性标志。治疗上目前缺乏标准化方案,主要采用高强度化疗联合造血干细胞移植(HSCT)进行治疗。明确的靶向治疗分子靶点仍未发现,新药SL-401已用于靶向治疗临床试验。     

自行消退的皮肤母细胞性浆细胞样树突细胞肿瘤

报告1例皮肤母细胞性浆细胞样树突细胞肿瘤。患者女,33岁,孕32+1周。全身多发紫红色结节及斑块1个月,无痛痒,外周血WBC及单核细胞均增多。皮肤科检查:躯干及四肢散在紫红色结节及斑块,表面光滑,质韧。皮损组织病理检查:真皮全层胶原束间单个核细胞浸润,可见核异形。骨髓穿刺示骨髓增生Ⅱ级,粒系0.27,红系0.29,单核细胞比例偏高,原幼单细胞占0.31,可见Auer小体。外周血涂片:单核细胞比例偏高,可见中幼粒细胞。免疫组化示CD56(+)及CD4部分(+)。诊断:皮肤母细胞性浆细胞样树突细胞肿瘤。患者未行任何治疗,剖宫产后皮损、血象及骨髓象均暂时好转。3个月后病情复发,进展为急性单核细胞白血病。血液科行CLAG方案化疗达到完全缓解。     

Alpha-interferon secreting blastic plasmacytoid dendritic cells neoplasm: a case report with histological, molecular genetics and long-term tumor cells culture studies

We report a new case of blastic plasmacytoid dendritic cell neoplasm (BPDCN) with extensive immunophenoptyping, genotyping (karyotype, array-comparative genomic hybridization, and fluorescent in situ hybridization), and long-term tumor cells culture. BPDCN is a very rare and aggressive disease clinically characterized by a skin revealing localization more or less rapidly disseminating to the bone marrow and other organs with or without and leukemia. The disease was initially phenotypically characterized by the expression of both CD4 and CD56 antigens, whereas lymphoid and myeloid lineage antigens were negative. A phenotypic link with alpha-interferon (IFN-I)-producing plasmacytoid dendritic cells was demonstrated. The data collected in this case report provide additional biological and genotypical data on tumor cells of BPDCN. This study confirms the capability of tumor cells to secrete IFN-I, demonstrated by biological IFN-I activity of cultured cells and immunohistochemical expression of Mx-1 protein. Although a common genetic profile involving chromosomes 5, 6, 9, 12, 13, and 15 has been identified, no specific genetic marker has been demonstrated that is specific to BPDCN. The demonstration of ETV6 gene deletion in this case deserves further investigations as a putative BPDCN marker.     

Blastic Plasmacytoid Dendritic Cell Neoplasm: Analysis of Clinicopathological Feature and Treatment Outcome of Seven Cases

BackgroundBlastic plasmacytoid dendritic cell neoplasm (BPDCN), which is derived from the precursor of plasmacytoid dendritic cells, is a rare and highly aggressive hematologic malignancy. It has only recently been recognized as a distinct entity. BPDCN characteristically has a predilection for cutaneous involvement.ObjectiveThe aim of this study was to describe the clinical and pathological features of BPDCN, and to review the treatment courses to analyze the prognosis and the optimal therapeutic approach.MethodsWe retrospectively reviewed seven BPDCN cases registered in the Samsung Medical Center database between January 2010 and December 2014.ResultsThe median age of the patients was 52 years (range, 18~79 years), and six patients were male. The clinical staging was as follows: skin (n=5), lymph node (n=6), bone marrow (n=4), and peripheral blood (n=2). The skin manifestations were bruise-like tumefaction (n=4), erythematous nodule (n=4), or multiple erythematous papules (n=1). The pathological evaluation revealed dense diffuse or nodular infiltration of neoplastic cells, which were positive for CD4, CD56, and CD123 in the immunohistochemical analysis. Six patients received multiagent chemotherapy as the first-line treatment, alone (n=4), or followed by stem cell transplantation (SCT, n=1) or concurrent radiotherapy (n=1). The median progression-free survival after the first-line treatment was 6 months (range, 2~12 months).ConclusionThree different skin manifestations were observed, with pathological features analogous to each other. All patients who received chemotherapy without SCT achieved partial or complete response but experienced relapse. Furthermore, they showed various clinical courses irrelevant to the cutaneous involvement.     

Bruise-like cutaneous lesions as the early presentation of blastic plasmacytoid dendritic cell neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and highly aggressive hematopoietic malignancy associated with a poor prognosis. It has been recognized to originate from precursors of plasmacytoid dendritic cells and has recently been established as a distinct entity. The most frequent clinical presentations are prominent skin lesions, followed by peripheral blood, bone marrow, and other organ involvement. Treatment outcomes are often disappointing due to a high relapse rate after chemotherapy and risk of leukemic dissemination. Herein, we report a case of BPDCN in a 41-year-old man who presented with cutaneous and nasopharyngeal lesions, and achieved a complete response after systemic chemotherapy. Dermatologists should be aware of BPDCN and its clinical presentations, as early diagnosis with appropriate treatment is crucial to improve its prognosis.     

Blastic plasmacytoid dendritic cell neoplasm

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare haematological malignancy that originates from the precursors of plasmacytoid dendritic cells. It commonly presents with findings isolated to the skin although it usually progresses to a leukaemic phase. It has a poor prognosis but is curable, particularly in younger patients treated with allogeneic bone marrow transplantation. We report a case of a 79‐year old man who had 6 months of progressive, asymptomatic BPDCN manifestations limited to the skin, before developing a leukaemic phase.     

Primary cutaneous blastic plasmacytoid dendritic cell neoplasm in a child: A challenging diagnosis and management

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive malignancy of the skin and hematopoietic system. There are few pediatric cases reported in the literature. Management of primary cutaneous BPDCN is challenging because, despite an apparently indolent clinical presentation, rapid dissemination with high mortality can occur. We describe a child with isolated cutaneous involvement who had a good response to chemotherapy as first‐line treatment of BPDCN.     

Lacking CD56 expression in a relapsing cutaneous blastic plasmacytoid dendritic cell neoplasm after allogeneic bone marrow transplantation: FISH analysis revealed loss of 11q

Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare entity characterized by a CD4+/CD56+/CD123+ immunophenotype and a fatal clinical course. The average survival of 12–14 months may be prolonged by allogeneic bone marrow transplantation (BMT). Objectives We report about a male patient who suffered from BPDCN with a typical histology and co‐expression of CD4/CD123 and a CD56 expression by 80% of the tumour cells. The cutaneous tumour relapse after chemotherapy and allogeneic BMT was completely negative for CD56. Methods We performed interphase fluorescence in situ hybridization (FISH) analysis of tumour tissue, asserved before and after BMT, using specific probes for chromosome 11, which encompass the CD56 gene region. Results The tumour cells revealed a partial loss of 11q as well as a monosomy of chromosome 11. Conclusion This case demonstrates for the first time that loss of CD56 expression can also occur as a secondary event after chemotherapy and BMT. In our case, DNA loss of 11q23 could be responsible for the negativity of 20% of tumour cells as observed before chemotherapy. However, the complete loss of CD56 expression in the relapsed tumour cannot be explained by the loss of 11q23 alone. Additional factors such as chemotherapy‐induced mutations might also have contributed.     

Blastic plasmacytoid dendritic cell neoplasm: is transplantation the treatment of choice?

Background  Blastic plasmacytoid dendritic cell neoplasm (BPDCN) represents the malignant counterpart derived from plasmacytoid dendritic cells. This rare entity is usually revealed and diagnosed on cutaneous lesions associated or not with a leukaemic component. The prognosis associated with BPDCN is very poor.
Objectives  To perform a retrospective review of BPDCN cases registered in the French Study Group on Cutaneous Lymphoma database from June 1995 to May 2008.
Methods  Forty-seven patients were included. Demographic data, initial staging, therapeutic management and outcome were recorded.
Results  The mean survival was 16·7 months (95% confidence interval 12·6–20·8). Only eight (17%) and one (2%) patients reached respectively 2 and 5 years of survival. Initial spreading of the disease did not represent, in this cohort, a reliable prognosis factor. The outcome was overall influenced by treatment provided. While radiation therapy, monochemotherapy or even polychemotherapy regimens did not significantly affect the course of the disease, the survival of bone marrow transplanted patients was significantly higher.
Conclusions  Despite the fact that BPDCN is often initially limited to the skin, only an aggressive initial therapy may improve the patients' prognosis. Local treatments, such radiation therapy, are definitively useless. Regardless of the initial extension of the disease, in our experience only bone marrow transplantation significantly improved the outcome.     

Blastic plasmacytoid dendritic cell neoplasm: a report of four cases and review of the literature

Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN), formerly known as agranular CD4⁺/CD56⁺ haematodermic neoplasm (CD4/CD56 HN), is a rare distinct form of lymphoma‐like entity known of dermatologists because of its marked predilection for cutaneous involvement, and its aggressive behaviour. Moreover, the association or the evolution to an acute leukaemia entity that still expresses CD4 and CD56 markers is almost systematic. This new described entity of ‘CD4⁺/CD56⁺ leukaemia’ or ‘leukaemia of plasmacytoid dendritic cell lineage’ has a poor prognostic and may lead to include haematopoietic stem cell transplantation in the treatment strategy as early as possible. Report of cases We report here four cases presenting with skin lesions and haematological signs. One of the patients underwent allogeneic stem cell transplantation, with a relapse‐free survival of 40 months. We discuss the diagnosis features as well as the treatment options. Conclusion A collaborative work between dermatologists and onco‐haematologists is essential to give patients the best chance of complete and long‐term response.